Joan Wise suffers from sickle cell anemia. As a child, "I saw the hospital more than I saw my home," she says. "I was always scared because I never knew when I was going to experience another crisis. It's like a bad toothache, or worse."
Pain has tormented her physically and mentally. She has difficulty keeping a job and medical insurance.
"I was never able to keep a job because of my illness. There is no fair warning as to when you are going to get sick," she says.
Ms. Wise, who lives in northwest Baltimore, has two sons, 12 and 19, who have the sickle cell trait. (A person can have the trait, but never get sickle cell anemia). Ms. Wise is a patient advocate for the National Association of Sickle Cell Anemia.
Sickle cell anemia is an inherited blood disorder that affects the shape and function of the red blood cells. The disease not only afflicts those of African-American decent but people of Greek, Turkish, Iranian, Puerto Rican, South and Central American decent as well.
One in every 12 black Americans has the sickle cell trait, which is inherited through a sickle cell gene from a parent. When both parents have the sickle cell trait, their children have a 25 percent chance that they will have sickle cell anemia, a 25 percent chance that they will have normal hemoglobin, and a 50 percent chance that they will have sickle cell trait.
The problem with sickle cell anemia is caused by a substance called hemoglobin which carries oxygen in the blood. Most people have normal hemoglobin, called hemoglobin A. People with sickle cell have an abnormal hemoglobin called hemoglobin S that causes the red blood cells to take on an abnormal, sickled shape.
The anemia creates episodes of excruciating pain called crises. This occurs when the abnormal red blood cells clog the blood vessels, and oxygen can't get to other cells in the body. As a result, symptoms begin to appear, including weakness, poor appetite, infections, yellowing of the eyes, and pain in the abdomen, chest and joints. This pain can occur at any time, and varies in duration and severity. The crisis lasts for a few hours or weeks and sometimes is so severe that the victim requires hospitalization.
The state of Maryland has developed an extensive newborn screening program. Whan an infant is born, full screening for sickle cell is performed. Before the implementation of the program, of children born with sickle cell disease, 5 percent died between the ages of 2 and 10 years old. Under the state program, the death rate has decreased in children under 2 years old, from 5 percent to zero.
Also, the number of infections has been reduced, but children with serious infections are those who were not taking their penicillin.
The testing is offered to infants after 24 hours of milk feeding and then again at four weeks of age. The state offers outreach clinics in Baltimore, Annapolis, Cheverly, Clinton, Cumberland, Easton, Elkton, Frederick, Hagerstown, LaPlata, Salisbury, Westminster and Wheaton. A team of doctors, nurses and social workers will make home visits. However, the newborn screening clinic follows a child intensely for three years after being diagnosed.
Dr. Susan Pani, director of the newborn screening program, recommends the following for parents of newborns who have sickle cell anemia:
*Special medication (penicillin) and immunization.
*Examination by a pediatric hematologist once a year.
*Keep all medical appointments.
*Attend parent support groups.
There are two organizations that play major roles in the lives of sickle cell patients. The National Association for Sickle Cell Disease, Maryland Chapter, is a non-profit organization, established in 1969, that seeks to improve the quality of life of individuals who have sickle cell anemia.
The chapter offers screening, treatments, education and counseling. Testing for sickle cell is free. Children between the .. ages of 6 and 14 can attend Camp Sunshine, which is for children with sickle cell disease. The camp is held every summer at Coppin State College and provides swimming, music, tutoring, art, dance and counseling.
The Association for Sickle Cell Services, Education, Research and Treatments is a community-based organization providing free testing, education, medical referral and counseling to sickle cell patients and other related hemoglobin variants. Anyone interested in free screening and counseling can contact ASSERT at 578-1800, or the National Association for Sickle Cell Disease, Maryland Chapter, at 837-3050.